Kayser-Fleischer rings

Kayser-Fleischer rings, sometimes shortened to K-F rings, are caused by copper deposition in the cornea and are a specific, clinical sign of Wilson disease.

Clinical presentation

They are usually brown or dark reddish in color. Early on they may need a slit lamp to be visible before they become visible to the naked eye. The ring can also be incomplete initially. Kayser-Fleischer rings are present in 95% of Wilson disease cases, including in nearly all cases with neurological manifestations . The density of the ring correlates well with the activity of the disease .

The rings are not pathognomonic for Wilson disease.

Pathology

Kayser-Fleischer rings are caused by direct copper deposition in the Descemet membrane of the cornea and are thought to be from epithelial cells absorbing copper from the aqueous humor .

Treatment and prognosis

After commencement of copper chelating agents (e.g. penicillamine), the size of the rings usually diminish and may eventually vanish altogether .

History and etymology

They were initially described by German ophthalmologist Bernhard Kayser (1869-1954) and German physician Bruno Fleischer (1848-1904) in 1902 and 1903 respectively .

Siehe auch:

• Morbus Wilson